Holmes-Adie syndrome, autoimmune hepatitis and celiac disease: a case report.

A 35-year-old female patient presented with the following symptoms of Holmes-Adie syndrome: photophobia,enlargement of the left pupil unresponsive to light, Achilles areflexia. The pilocarpine test was positive. No tumor or other neurological abnormality was found. She had a 19-year history of autoimmune hepatitis. Flares up were observed following each 3 deliveries. At age of 31 she presented with diarrhea and weight loss. Abdominal tumor was detected by ultrasound. The surgically removed tumor was histologically a benign mesenteric multicystic lymphangioma.Simultaneously, celiac disease was diagnosed.Gluten-free diet resulted in a significant improvement of celiac disease,but not of autoimmune hepatitis. Autonomic neuropathy was proven by standard cardiovascular tests. The patient was a homozygous carrier for HLA DQ2 antigen characteristic for celiac disease and heterozygous for HLA DR3 B8 frequent in autoimmune liver diseases. Our novel observation on association of Holmes-Adie syndrome with autoimmune hepatitis and celiac disease is suggestive for a common immunological background for all three entities present in a patient with mesenteric multicystic lymphangioma.

A hypothetical immunemediated unifying mechanism for the Holmes-Adie syndrome.

The Holmes-Adie syndrome consists of pupillotonia, arreflexia and autonomic dysfunction. Some explanations for these different symptoms have been attempted, centred upon neuropathological and electrophysiological findings. A hypothetical immunemediated mechanism, as in the Guillain Barré syndrome, is presented in this paper for explaining the three chief symptoms of the syndrome.

A search for serum antibodies in Adie's syndrome.

Serum specimens from a large number of patients with Adie's syndrome were checked for virus antibody levels: Measles hemagglutination inhibition (HI), parainfluenza types I, II and III (HI), Epstein-Barr immunofluorescence, Mumps complement fixation (CF), Adenovirus (CF), Varicella-Zoster (CF), Herpes simplex (CF), Cytomegalovirus (CF), Mycoplasma hominis (CF), Toxoplasma gondii passive hemagglutination. These antibody levels were compared with specimens from a control group of similar age distribution and sex ratio. No statistically significant differences between the patients with Adie's syndrome and the control group could be demonstrated. This observation offers some indirect support to Harriman's idea that Adie's syndrome is not due to a viral ciliary ganglionitis but rather to an indolent neuronal degeneration in the ciliary and dorsal root ganglia.

A hypothetical immunemediated unifying mechanism for the holmes-Adie syndrome

A síndrome de Holmes-Adie consiste de pupila tônica, arreflexia patelar e disfunçäo autômica. As explicaçöes desses diferentes síntomas têm-se baseado em achados neuropatológicos e eletrofisiológicos. Neste trabalho apresenta-se uma hipótese imunológica para a fisiopatogenia da síndrome, de modo semelhante à da síndrome de Guillain-Barré